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 2,562 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adrenocortical causes of hypertension are established by examining the mineralocorticoid hormones produced in the zona glomerulosa and zona fasciculata. In the zona glomerulosa, aldosterone excess leads to hypertension, hypokalemia, and suppressed plasma renin activity, with increased concentrations of urinary aldosterone (either as the 18-glucuronide or free aldosterone) as an index of its production. Identifying a tumor by computed tomography scan verifies the diagnosis of a correctable lesion. If no tumor is found, several maneuvers are used to identify primary adrenal hyperplasia, a disorder with autonomous aldosterone production, for which reduction of adrenal mass is curative. The zona fasciculata has two major pathways: the 17-deoxy pathway, where deoxycorticosterone (DOC) and corticosterone are the significant steroids, and the 17-hydroxy pathway, which leads to cortisol production. Tumors of the 17-deoxy pathway, DOC-producing adenomas, have increased concentrations of DOC and its precursor steroids, normal concentrations of cortisol, and suppression of aldosterone production secondary to suppression of the renin system. Two enzymatic defects in the zona fasciculata, 11 beta- and 17 alpha-hydroxylase deficiency, can be first readily identified by the virilization in the former, hypogonadal features in the latter. Steroid patterns are diagnostic. DOC is produced in excess in both deficiencies and is the cause of the hypertension. Deficient or impaired 11 beta-hydroxy steroid dehydrogenase in the apparent mineralocorticoid excess syndrome or after licorice ingestion retards the conversion of cortisol to inactive cortisone in the kidney, leading to mineralocorticoid hypertension; this leads to suppression of the renin system and subsequently of aldosterone.
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PMID:Steroid characteristics of mineralocorticoid adrenocortical hypertension. 191

Virilizing adrenal adenomas are rare, and Leydig cell adenomas of the adrenal rarer still. Our patient, a 60-year-old virilized woman, was found to have a Leydig cell adenoma. Virilization associated with normal 17-ketosteroid and elevated testosterone levels necessitates excluding an adrenal cause. Although one might expect high testosterone levels to suppress gonadotropins, this is not the case. In addition to other studies recommended for assessing the incidentally discovered adrenal mass, we would add a testosterone assay.
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PMID:Virilization due to a Leydig cell adrenal adenoma. 283 7

A 31-year-old woman who presented with virilization was found to have elevated plasma testosterone and increased urinary 17-KS levels. The apparent source of androgens was detected and defined by CT scanning as a right-sided adrenal mass lesion, which was successfully resected and which proved to be an adrenocortical carcinoma.
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PMID:Virilizing adrenocortical carcinoma: role of computed tomography. 724 62

We describe a rare androgen and desoxycorticosterone (DOC)-secreting adrenal tumor in a non-Cushingoid 14 year-old Haitian girl with secondary amenorrhea, hypertension and virilization. Her steroid pattern simulated an 11 beta-hydroxylation defect with notable elevation of adrenal androgens, 11-desoxycortisol (S), DOC, 17 alpha-hydroxyprogesterone and pregnenelone. Exogenous ACTH stimulated steroidogenesis. A CAT scan unfortunately failed to delineate an adrenal mass. Dexamethasone (DEX) was administered, therefore, which partially suppressed androgen levels, reduced DOC and S by 80% and 82% respectively, and normalized blood pressure. Nevertheless, the response to glucocorticoid was incomplete and an MRI was obtained, which revealed a right adrenal tumor. Post surgery, the patient promptly resumed menses and became normotensive. This case illustrates that ACTH and DEX cannot reliably differentiate tumor from hyperplasia, whereas the simultaneous increase of delta 4 and delta 5 steroids, present here, may favor a tumor. This case also allows speculation that the hypersecretion of DOC may result from inhibition of 11 beta-hydroxylase activity by excess androgens. The importance of appropriate imaging for diagnosis is underscored.
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PMID:Hypertension and virilization caused by a unique desoxycorticosterone- and androgen-secreting adrenal adenoma. 1039 70

Primary neoplasms of the adrenal cortex are rare in children and differ significantly in epidemiology, clinical characteristics, and biologic features from their counterparts in adults. In children, the inclusive term adrenocortical neoplasm is applied because adrenal adenoma and adrenal carcinoma may be difficult to distinguish histopathologically. Pediatric adrenocortical neoplasms typically occur before 5 years of age, affect young girls more commonly than boys, and are associated with hemihypertrophy and Beckwith-Wiedemann and Li-Fraumeni syndromes. Most children with an adrenocortical neoplasm present with signs and symptoms of endocrine abnormality, including virilization and Cushing syndrome. Cross-sectional imaging studies typically demonstrate a large, circumscribed, predominantly solid suprarenal mass with variable heterogeneity due to hemorrhage and necrosis. Calcification is not uncommon. Local invasion and metastases to the lungs, liver, and regional lymph nodes may be present at diagnosis. When friable tumor thrombus extends into the inferior vena cava, it poses a high risk of pulmonary embolization. The finding of increased retroperitoneal fat due to hypercortisolism on computed tomographic and magnetic resonance images of children with an adrenal mass favors the diagnosis of adrenocortical neoplasm. Surgical resection is the mainstay of therapy, with chemotherapy used for patients with metastases or persistent elevated hormone levels following surgery. Patients younger than 5 years with aggressive adrenocortical neoplasms fare better than older children.
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PMID:Adrenocortical neoplasms in children: radiologic-pathologic correlation. 1046 5

We report a case of a virilized 59-yr-old woman with elevated serum testosterone levels and bilateral macronodular adrenal hyperplasia. The patient underwent laparoscopic right adrenalectomy, after which the elevated testosterone level transiently normalized. The immediate postoperative depression of the testosterone level suggested that the process was driven by gonadotropins that were suppressed by the stress of surgery. The excised right adrenal mass contained testosterone by immunohistochemistry and LH receptor mRNA by in situ hybridization. The recurrence of hyperandrogenemia suggested that the enlarged left adrenal was also secreting testosterone. The serum testosterone level increased in response to im injection of human chorionic gonadotropin, suggesting control by aberrant LH receptors. Injection of leuprolide acetate (7.5 mg im) to suppress LH levels resulted in normalization of the testosterone level 12 d later that persisted for several weeks. Ectopic receptors mediating Cushing's syndrome have been described in several cases of bilateral adrenal hyperplasia and adrenal adenoma. This is the first case to our knowledge in which pure androgen overproduction in adrenal hyperplasia has been shown to be controlled by LH receptors. In our patient, the control of androgen secretion by LH may explain the postmenopausal onset of virilization and the transient postoperative normalization of the serum testosterone level.
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PMID:Virilization in bilateral macronodular adrenal hyperplasia controlled by luteinizing hormone. 1251 32

[Iodine-131]6-beta-iodomethylnorcholesterol (NP-59) visualization of adrenocortical carcinoma is unusual. We describe a 17-year-old female with virilization and elevated plasma testosterone, dehydroepiandrosterone sulfate (DHEAS) and aldosterone. Magnetic resonance imaging disclosed a 9-cm right adrenal mass. NP-59 adrenal scanning displayed unilateral uptake of tracer and no visualization of the contralateral adrenal gland. Exploratory laparotomy revealed adrenocortical carcinoma. Subsequent immunohistochemical studies confirmed that the tumor was capable of producing a mixture of steroids, including testosterone, DHEAS and aldosterone. Visualization of an adrenal tumor on NP-59 scintiscan is an unusual finding, which cannot exclude the possibility of malignancy.
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PMID:Unusual visualization of an adrenal carcinoma on NP-59 scintiscan. 1661 15

A 23-month-old boy with persistent acne since 6 months of age had signs of virilization and accelerated growth. Hormone evaluation indicated increased levels of dehydroepiandrosterone (DHEA) and testosterone. Ultrasound and abdominal computed tomographic scan revealed a large adrenal mass consistent with an adrenocortical tumor. The patient underwent surgical excision of the well-encapsulated tumor with normalization of his hormones and no subsequent recurrence. Although rare, childhood adrenocortical tumors have a poor prognosis, with the majority of tumors having regional and metastatic disease. Because early diagnosis and complete surgical excision improve prognosis, children with refractory infantile acne should be evaluated for signs of virilization and accelerated growth. Laboratory evaluation should include luteinizing hormone, follicle-stimulating hormone, total and free testosterone cortisol, DHEA, and its sulfate (DHEA-S). Elevated levels of DHEA and DHEA-S should prompt an aggressive diagnostic evaluation for an adrenocortical tumor.
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PMID:Infantile acne as the initial sign of an adrenocortical tumor. 1709 83

Adrenal incidentaloma is not infrequent and can be found in hirsute women. We report a case of a 54-year-old woman with amenorrhea and hirsutism of abrupt onset and mild signs of virilization that had an adrenal incidentaloma coexisting with ovarian hyperthecosis. Basal total and free testosterone were 191 ng/dL and 179 pmol/L. Pelvic ultrasonography disclosed a right ovary with 10.3 cc and a left ovary with 9.8 cc without nodules or cysts, and computerized tomography of the abdomen disclosed a normal right adrenal gland. On the left adrenal gland a solid nodule with 0.8 cm was seen. After GnRHa administration, total testosterone was 23 ng/dL and free testosterone was 17 pmol/L. In view of a suppression of testosterone by GnRHa, the patient was submitted to a hystero-oophorectomy by laparoscopy. Symmetrically enlarged ovaries were seen. No tumor was apparent. Histology showed hyperthecosis, with foci of luteinized stromal cells. Only atretic follicles were detected. No hilar cell hyperplasia was seen. In conclusion, the presence of an adrenal mass in a hirsute woman can lead to a wrong diagnosis. In this case the suppression GnRHa test was fundamental to determine the origin of hyperandrogenemia.
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PMID:Ovarian hyperthecosis in the context of an adrenal incidentaloma in a postmenopausal woman. 1908 8

Adrenal myelolipoma is a rare, benign and usually hormonally inactive tumour. We report a case of a young female who presented with hormonally active tumour causing virilization and associated type-2 Diabetes mellitus. Laparoscopic adrenalectomy was done after CT finding of a large left adrenal mass that was producing large amounts of androgens. Adrenal myelolipoma with cortical hyperplasia was diagnosed on histopathological examination. Her diabetes progressively regressed after the removal of tumour and glucose tolerance remained normal up to 1 year of follow-up after surgery and there was no recurrence of tumour. She also had a lump in her breast which proved to be a fibroadenoma. We report this case due to its rarity, multiplicity of tumours and adrenal cortical hyperplasia-presenting as an unusual cause of severe virilization.
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PMID:Virilization with adrenal myelolipoma, adrenal hyperplasia, and fibroadenoma of breast. 2120 49


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